183 Triple-Hit B-Cell Lymphoma: Case Report of an Elderly Patient Achieving Complete Remission. (11th January 2018)

• Record Type: Journal Article
• Authors: Annunziata, Joseph; Balog, Anna; Tang, Bang
• Is Part Of: American journal of clinical pathology. Volume 149(2018)Supplement 1
• Page Start: S78
• Page End: S78
• Publication Date: 2018-01-11
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqx121.182
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract: Large B-cell lymphoma with rearrangements of MYC, BCL2, and BCL6, so-called "triple-hit" lymphoma, now designated as high-grade B-cell lymphoma (HGBL), with MYC and BCL2 and/or BCL6 rearrangements per World Health Organization classification, is an aggressive, rare malignancy typically affecting older adults. We report a case in an 87-year-old Caucasian man who presented with a rapidly enlarging, 14.9 cm chest wall mass. MRI also showed chest and axillary lymphadenopathy and a lesion in left humeral head. Histologic sections of chest mass demonstrated an atypical lymphoid infiltrate with intermediate to large cells showing stippled to vesicular chromatin, brisk mitoses and punctate necrosis. Immunohistochemistry showed positivity for CD20, CD10, BCL-2, and BCL-6 in lymphoid cells. In situ hybridization (ISH) for kappa and lambda demonstrated kappa light chain restriction. MIB-1 was high (80%). Fluorescence ISH demonstrated MYC, BCL-2, and BCL-6 rearrangements. The diagnosis of HGBL, with rearrangements of MYC, BCL2, and BCL6, was rendered. The patient received chemotherapy with bendamustine and rituximab, and has been in remission for 7.5 months. Triple-hit lymphomas are not frequently reported; thus, the incidence is unknown. However, it is important to document this entity as it has distinct prognostic and therapeutic implications. Currently, the challenge is how to adequately screen for this entity. Many institutions perform molecular studies on any B-cell lymphoma showing features intermediate between DLBCL and BL, a MIB-1 >90%, or any DLBCL refractory to therapy. Others suggest limiting studies to cases of germinal center origin and/or high-grade morphology or to cases with > 40% MYC+ cells. MYC immunohistochemistry can also help identify double-expressor lymphomas, defined by coexpression of MYC and BCL2 by IHC, which are associated with a worse prognosis than other LBCLs, but may not behave as aggressively as double/triple-hit lymphomas (HGBLs).